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pediatric heart failure

Matteo Di Nardo, Graeme MacLaren, Marco Marano, Corrado Cecchetti, Paola Bernaschi, Antonio Amodeo
Extracorporeal life support (ECLS) is an important device in the management of children with severe refractory cardiac and or pulmonary failure. Actually, two forms of ECLS are available for neonates and children: extracorporeal membrane oxygenation (ECMO) and use of a ventricular assist device (VAD). Both these techniques have their own advantages and disadvantages. The intra-aortic balloon pump is another ECLS device that has been successfully used in larger children, adolescents, and adults, but has found limited applicability in smaller children...
2016: Frontiers in Pediatrics
Lijiang Ma, Wendy K Chung
Group 1 pulmonary hypertension or pulmonary arterial hypertension (PAH) is a rare disease characterized by proliferation and occlusion of small pulmonary arterioles, leading to progressive elevation of pulmonary artery pressure and pulmonary vascular resistance, and right ventricular failure. Historically it has been associated with a high mortality rate, although over the last decade, treatment has improved survival. PAH includes idiopathic PAH (IPAH), heritable PAH (HPAH), and PAH associated with certain medical conditions...
October 22, 2016: Journal of Pathology
K M Olsson, M Halank, B Egenlauf, D Fistera, H Gall, C Kaehler, K Kortmann, T Kramm, M Lichtblau, A Marra, C Nagel, A Sablotzki, H-J Seyfarth, D Schranz, S Ulrich, M M Hoeper, T J Lange
The 2015 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension are also valid for Germany. The guidelines contain detailed recommendations for the targeted treatment of pulmonary arterial hypertension (PAH). However, the practical implementation of the European Guidelines in Germany requires the consideration of several country-specific issues and already existing novel data. This requires a detailed commentary to the guidelines, and in some aspects an update already appears necessary. In June 2016, a Consensus Conference organized by the PH working groups of the German Society of Cardiology (DGK), the German Society of Respiratory Medicine (DGP) and the German Society of Pediatric Cardiology (DGPK) was held in Cologne, Germany...
October 2016: Deutsche Medizinische Wochenschrift
Stephen Robert Daniels
Hypertension in children and adolescents is associated with both short and long-term abnormalities in the cardiovascular system. Ultimately, these changes can lead to myocardial infarction, stroke, congestive heart failure and chronic renal disease, all of which are associated with substantial morbidity and mortality.In the short term, the best evidence that hypertension is adversely impacting the heart and vasculature is the development of left ventricular hypertrophy, increased vascular stiffness and endothelial dysfunction...
September 2016: Journal of Hypertension
Carolina López, Andrés José Alcaraz, Blanca Toledo, Lucía Cortejoso, Maite Augusta Gil-Ruiz
OBJECTIVE: Patients in PICUs frequently present hypochloremic metabolic alkalosis secondary to loop diuretic treatment, especially those undergoing cardiac surgery. This study evaluates the effectiveness of acetazolamide therapy for metabolic alkalosis in PICU patients. DESIGN: Retrospective, observational study. SETTING: A tertiary care children's hospital PICU. PATIENTS: Children receiving at least a 2-day course of enteral acetazolamide...
September 29, 2016: Pediatric Critical Care Medicine
Satoshi Masutani, Seiko Kuwata, Clara Kurishima, Yoichi Iwamoto, Hirofumi Saiki, Masaya Sugimoto, Hirotaka Ishido, Hideaki Senzaki
OBJECTIVE: The details of the ventricular-vascular dynamics of heart failure with preserved ejection fraction (HFpEF) in children remain poorly understood. We tested the hypothesis that pediatric HFpEF patients have ventricular systolic, diastolic, and arterial stiffening at rest as well as impaired reserve function associated with coronary supply/demand imbalance. METHODS: We studied the ventricular pressure-area relationship in 22 pediatric HFpEF patients and 22 control subjects before and after dobutamine infusion and during abdominal compression...
September 30, 2016: International Journal of Cardiology
Saro H Armenian, Melissa M Hudson, Ming Hui Chen, Steven D Colan, Lanie Lindenfeld, George Mills, Aida Siyahian, Sarah Gelehrter, Ha Dang, Wendy Hein, Daniel M Green, Leslie L Robison, F Lennie Wong, Pamela S Douglas, Smita Bhatia
BACKGROUND: Anthracyclines are widely used in the treatment of childhood cancer. One of the well-recognized side-effects of anthracycline therapy is dose-dependent cardiomyopathy that may progress to heart failure (HF) years after completion of cancer-directed therapy. This study will evaluate the efficacy of low-dose beta-blocker (carvedilol) for HF risk reduction in childhood cancer survivors at highest risk for HF. The proposed intervention has the potential to significantly reduce chronic cardiac injury via interruption of neurohormonal systems responsible for left ventricular (LV) remodeling, resulting in improved cardiac function and decreased risk of HF...
October 4, 2016: BMC Cardiovascular Disorders
Wanli Li, Xinjiang An, Mingyu Fu, Chunli Li
Pneumonia refers to lung inflammation caused by different pathogens or other factors, and is a common pediatric disease occurring in infants and young children. It is closely related to the anatomical and physiological characteristics of infants and young children and is more frequent during winter and spring, or sudden changes in temperature. Pneumonia is a serious disease that poses a threat to children's health and its morbidity and mortality rank first, accounting for 24.5-65.2% of pediatric inpatients...
October 2016: Experimental and Therapeutic Medicine
Glenn J R Whitman
Use of extracorporeal membrane oxygenation (ECMO), one of an increasing variety of mechanical circulatory support strategies, was first used close to 50 years ago. For decades, it was mostly applied to the pediatric population. However, during the past several years, its use has dramatically increased as therapy for pulmonary and cardiac failure in the adult. In particular, ECMO is being used more and more for postcardiotomy shock. Unfortunately, despite its increased application in this setting, improved outcomes have been hard to come by...
August 31, 2016: Journal of Thoracic and Cardiovascular Surgery
Guang Song, Jing Zhang, Weidong Ren, Ying Li, Ke Zhou
BACKGROUND: Coronary artery fistula (CAF) is a rare cardiac anomaly. Application of transthoracic echocardiography (TTE) is not fully illustrated in pediatric period. Meanwhile, the treatment strategy of CAF is still a controversial issue. CASE PRESENTATION: Five cases of CAF with different types were presented. We also retrospectively reviewed 32 records of CAF in our institution from May 2001 to January 2015, including cardiac murmurs, symptoms, TTE diagnoses, complications, other anomalies, treatment and outcome...
2016: SpringerPlus
Stephen Robert Daniels
Hypertension in children and adolescents is associated with both short and long-term abnormalities in the cardiovascular system. Ultimately, these changes can lead to myocardial infarction, stroke, congestive heart failure and chronic renal disease, all of which are associated with substantial morbidity and mortality.In the short term, the best evidence that hypertension is adversely impacting the heart and vasculature is the development of left ventricular hypertrophy, increased vascular stiffness and endothelial dysfunction...
September 2016: Journal of Hypertension
James H Hertzog, Thomas E Pearson, Marc A Priest, Ellen Spurrier, Ryan R Davies
Ventricular assist devices (VADs) for the mechanical support of cardiac failure are being used more frequently in children of increasingly younger age. These children have significant and multiple medical comorbidities, and their length of hospital stay has been increasing. As this population of hospitalized VAD-supported children increases, so does the possibility of their need for interfacility transport for specialized diagnostic or therapeutic procedures. Reports on such transports are limited to 3 children who underwent scheduled elective transfers...
September 2016: Air Medical Journal
Rogatus Kabyemera, Neema Chami, Neema Kayange, Respicius Bakalemwa, Antke Zuechner, Tumaini Mhada, Gustave Buname, Adolfine Hokororo, Johannes Kataraihya
Upper airway obstruction (UAO) due to adenotonsillar hypertrophy represents one of the rare causes of pulmonary hypertension in children. We report a case of adenotonsillar hypertrophy, managed at pediatric and otorhinolaryngology departments in Bugando Medical Centre (BMC), northwestern Tanzania, with complete remission of symptoms of pulmonary hypertension following adenotonsillectomy. A 17-month-old boy presented with difficulty breathing, dry cough, and noisy breathing since 1 year. He had facial and lower limb oedema with a pan systolic murmur at the tricuspid area, fine crepitations, and tender hepatomegaly...
2016: Case Reports in Pediatrics
Miguel Angel Palomero-Rodríguez, Héctor Chozas de Arteaga, Yolanda Laporta Báez, Jesús de Vicente Sánchez, Pascual Sanabria Carretero, Pilar Sánchez Conde, Antonio Pérez Ferrer
BACKGROUND: Over the last years, we have used a flow-inflating bag circuit with a nasotracheal or nasopharyngeal tube as an interface to deliver effective CPAP support in infants ("Mapleson D CPAP system"). The primary goal of this study was to assess the usefulness of the "Mapleson D CPAP system" for weaning of mechanical ventilation (MV) in infants who received MV over 24 h. MATERIALS AND METHODS: All infants who received MV for more than 24 h in the last year were enrolled in the study...
September 2016: Lung India: Official Organ of Indian Chest Society
Reena Khantwal Joshi, Neeraj Aggarwal, Mridul Agarwal, Veronique Dinand, Raja Joshi
OBJECTIVE: To delineate risk factors for failure of extubation in the operating room among pediatric cardiac surgery patients. DESIGN: Prospective, observational study. SETTING: Single center, tertiary care, teaching hospital. PARTICIPANTS: The study comprised 448 congenital cardiac surgery patients who were enrolled for intended extubation in the pediatric cardiac operating room over 5 years. INTERVENTIONS: The airways of enrolled patients were extubated in the operating room if predetermined suitability criteria were met...
June 16, 2016: Journal of Cardiothoracic and Vascular Anesthesia
Hala Mahmoud Koura, Neamat M Abdalla, Mona Hamed Ibrahim, Maha M A Abo Hashish, Sherif Mohamed Zaki
BACKGROUND: B-type natriuretic peptide (BNP) levels are elevated in children with congenital heart disease involving a left-to-right shunt (LRS) and are also raised in dilated cardiomyopathy (DCM). As far as we know, there are few reports in the literature comparing the change of the NT-proBNP in LRS and DCM especially in the pediatric age group. OBJECTIVES: The aim of the study was to compare the changes of the NT-proBNP in pediatric patients with LRS and DCM. Correlation between the levels of NT-proBNP and the echocardiographic parameters in both groups was determined...
June 2016: Iranian Journal of Pediatrics
Rafal Ploski, Malgorzata Rydzanicz, Tomasz M Ksiazczyk, Maria Franaszczyk, Agnieszka Pollak, Joanna Kosinska, Ewa Michalak, Piotr Stawinski, Lidia Ziolkowska, Zofia T Bilinska, Bozena Werner
Restrictive cardiomyopathy is a rare form of pediatric cardiac disease, for which the known genes include MYH7, TNNT2, TNNI3, ACTC1, and DES. We describe a pediatric proband with fatal restrictive cardiomyopathy associated with septal hypertrophy and compound heterozygosity for TNNC1 mutations (NM_003280: p.A8V [c.C23T] and p.D145E [c.C435A]). This association between restrictive cardiomyopathy and TNNC1 mutations was strengthened by prospective observations on the second pregnancy in the family which revealed, in the presence of the same TNNC1 genotype, prenatally diagnosed hypertrophic cardiomyopathy which evolved into restrictive cardiomyopathy, heart failure and death at the age of 9 months...
September 8, 2016: American Journal of Medical Genetics. Part A
Yasunari Niimi
Intracranial arteriovenous shunts (ICAVSs) in young children are characterized with frequent high flow fistulas. In association of high flow fistulas and physiological condition of the developing brain and heart, each disease tends to present at a certain age with unique symptoms. Vein of Galen aneurysmal malformation (VGAM) and dural sinus malformation (DSM) with arteriovenous (AV) shunts tend to present in neonate with high output cardiac failure. In infancy, VGAM, pial AVF and infantile dural AVF tend to present with hydrodynamic disorder such as macrocephaly, ventriculomegaly, prominent facial veins, and developmental delay...
September 7, 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
J Vockley, J Charrow, J Ganesh, M Eswara, G A Diaz, E McCracken, R Conway, G M Enns, J Starr, R Wang, J E Abdenur, J Sanchez-de-Toledo, D L Marsden
Long-chain fatty acid oxidation disorders (LC-FAOD) can cause cardiac hypertrophy and cardiomyopathy, often presenting in infancy, typically leading to death or heart transplant despite ongoing treatment. Previous data on triheptanoin treatment of cardiomyopathy in LC-FAOD suggested a clinical benefit on heart function during acute failure. An additional series of LC-FAOD patients with critical emergencies associated with cardiomyopathy was treated with triheptanoin under emergency treatment or compassionate use protocols...
August 27, 2016: Molecular Genetics and Metabolism
Valentina Favalli, Eliana Disabella, Mariadelfina Molinaro, Marilena Tagliani, Anna Scarabotto, Alessandra Serio, Maurizia Grasso, Nupoor Narula, Carmela Giorgianni, Clelia Caspani, Monica Concardi, Manuela Agozzino, Calogero Giordano, Alexandra Smirnova, Takahide Kodama, Lorenzo Giuliani, Elena Antoniazzi, Riccardo G Borroni, Camilla Vassallo, Filippo Mangione, Laura Scelsi, Stefano Ghio, Carlo Pellegrini, Marialuisa Zedde, Laura Fancellu, GianPietro Sechi, Antonello Ganau, Stefania Piga, Annarita Colucci, Daniela Concolino, Maria Teresa Di Mascio, Danilo Toni, Marina Diomedi, Claudio Rapezzi, Elena Biagini, Massimiliano Marini, Maurizia Rasura, Maurizio Melis, Antonia Nucera, Donata Guidetti, Michelangelo Mancuso, Umberto Scoditti, Pamela Cassini, Jagat Narula, Luigi Tavazzi, Eloisa Arbustini
BACKGROUND: Anderson-Fabry disease (AFD) is a rare X-linked lysosomal storage disease, caused by defects of the alpha-galactosidase A (GLA) gene. AFD can affect the heart, brain, kidney, eye, skin, peripheral nerves, and gastrointestinal tract. Cardiology (hypertrophic cardiomyopathy), neurology (cryptogenic stroke), and nephrology (end-stage renal failure) screening studies suggest the prevalence of GLA variants is 0.62%, with diagnosis confirmation in 0.12%. OBJECTIVES: This study sought to expand screening from these settings to include ophthalmology, dermatology, gastroenterology, internal medicine, pediatrics, and medical genetics to increase diagnostic yield and comprehensively evaluate organ involvement in AFD patients...
September 6, 2016: Journal of the American College of Cardiology
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